Paroxysmal nocturnal hemoglobinuria in childhood and adolescence--a retrospective analysis of 18 cases.
Indian J Pediatr
;
2008 Jun; 75(6): 575-8
Artigo
em Inglês
| IMSEAR
| ID: sea-84843
ABSTRACT
OBJECTIVE:
To assess the clinical and hematological profile of PNH in children.METHODS:
Clinical and laboratory features of children with PNH diagnosed in the past six years at our centre were reviewed. Various investigations done included a complete blood count and peripheral smear examination, plasma hemoglobin, urine hemosiderin, acid ham test, sucrose lysis test, immunophenotyping of erythrocytes by sephadex column gel card and of granulocytes by flow cytometry. There were 18 children with a marked male predominance (M 14 F 4).RESULTS:
Pallor, jaundice, dark urine and bleeding manifestations were the major presenting complaints. One girl suffered an arterial stroke. All children had cytopenia in at least one cell line. Children were treated with danazol, stanazolol, prednisolone and cyclosporin A variously. Overall response rate was 61%. Children with classical PNH performed slightly better with response rates of 66% (6/9) as compared to aplastic anemia-PNH group which has a response rate of 55% (5/9). Amongst various variables only danazol correlated with better response (p=0.029).CONCLUSION:
PNH is an uncommon disease in children and should be included in the differential diagnosis of children presenting with cytopenia.
Texto completo:
DisponíveL
Índice:
IMSEAR (Sudeste Asiático)
Assunto principal:
Trombose
/
Feminino
/
Humanos
/
Masculino
/
Criança
/
Fatores Sexuais
/
Taxa de Sobrevida
/
Estudos Retrospectivos
/
Adolescente
/
Resultado do Tratamento
Tipo de estudo:
Estudo observacional
Idioma:
Inglês
Revista:
Indian J Pediatr
Ano de publicação:
2008
Tipo de documento:
Artigo
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